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中华妇幼临床医学杂志(电子版) ›› 2020, Vol. 16 ›› Issue (03) : 358 -361. doi: 10.3877/cma.j.issn.1673-5250.2020.03.016

所属专题： 文献；

论著

Mayer-Rokitansky-Kuster-Hauser综合征患者的超声特征
伍婷1, 庞敏1, 杨帆1, 罗红1,†()   
  1. 1. 四川大学华西第二医院超声科、出生缺陷与相关妇儿疾病教育部重点实验室，成都　610041
  • 收稿日期:2020-01-10 修回日期:2020-04-22 出版日期:2020-06-01
  • 通信作者: 罗红

Ultrasonic features of patients with Mayer-Rokitansky-Kuster-Hauser syndrome

Ting Wu1, Min Pang1, Fan Yang1, Hong Luo1,†()   

  1. 1. Department of Ultrasound, Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
  • Received:2020-01-10 Revised:2020-04-22 Published:2020-06-01
  • Corresponding author: Hong Luo
  • About author:
    Corresponding author: Luo Hong, Email:
  • Supported by:
    Popularization and Application Project of Sichuan Provincial Health and Family Planning Commission(17PJ415); Science and Technology Huimin Technology Research and Development Project of Chengdu Science and Technology Bureau(2014-HM01-00042-SF); New Bud Research Project of West China Second University Hospital, Sichuan University(KX007, KX065)
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伍婷, 庞敏, 杨帆, 罗红. Mayer-Rokitansky-Kuster-Hauser综合征患者的超声特征[J/OL]. 中华妇幼临床医学杂志(电子版), 2020, 16(03): 358-361.

Ting Wu, Min Pang, Fan Yang, Hong Luo. Ultrasonic features of patients with Mayer-Rokitansky-Kuster-Hauser syndrome[J/OL]. Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition), 2020, 16(03): 358-361.

目的

探讨Mayer-Rokitansky-Kuster-Hauser(MRKH)综合征患者腹部超声检查的影像学特点。

方法

选择2000年至2018年，因"进入青春期后，无月经初潮"于四川大学华西第二医院就诊，而被诊断为MRKH综合征的17例患者为研究对象。回顾性分析其腹部超声的影像学特征。本研究遵循的程序，符合2013年修订的《世界医学协会赫尔辛基宣言》。

结果

这17例患者的年龄为(18±5)岁，染色体核型分析及血清性激素水平检查结果均正常。其中，15例被诊断为MRKH综合征Ⅰ型(88.2%)，其余2例被诊断为MRKH综合征Ⅱ型(11.8%)。其超声检查结果均未查见阴道气体线回声。其中，1例(5.9%)无子宫，6例(35.3%)正常发育子宫，3例(17.6%)正常宫体、无宫颈，2例(11.8%)单侧始基子宫、无宫颈，4例(23.5%)双侧始基子宫，1例(5.9%)双子宫无宫颈。10例(58.8%)子宫内膜正常，3例(17.6%)附件区见囊性占位，2例(11.8%)患者合并重复肾畸形。

结论

无阴道气体线声像图，是MRKH综合征患者的主要超声表现，超声检查时，还应同时注意患者有无合并其他畸形。腹部超声检查，可为该病患者的准确诊断及分型提供影像学依据。

关键词: 苗勒管发育不全, 阴道闭锁，先天性的, 女(雌)性泌尿生殖系统疾病, 超声检查, 月经初潮, 少女
Objective

To explore features of abdominal ultrasonic images of patients with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome.

Methods

From 2000 to 2018, a total of 17 patients with MRKH syndrome diagnosed in West China Second University Hospital, Sichuan University due to " no menarche after entering puberty" , were selected as research subjects. Their abdominal ultrasound diagnosis results were analyzed retrospectively. The procedure followed in this study was in line with the World Medical Association Declaration of Helsinki revised in 2013.

Results

The average age of 17 patients was (18±5) years old, and results of karyotype analysis and serum sex hormone levels examination were normal. Among them, 15 cases were diagnosed as MRKH syndrome type Ⅰ (88.2%), and the remaining 2 cases were diagnosed as MRKH syndrome type Ⅱ (11.8%). Ultrasound examination results showed that no vaginal gas line echo were found in all patients. Among them, 1 case (5.9%) had no uterus, 6 cases (35.3%) had a normal developing uterus, 3 cases (17.6%) had a normal uterus without cervix, 2 cases (11.8%) had unilateral primordial uterus without cervix, 4 cases (23.5%) had bilateral primordial uterus, 1 case (5.9%) had double uterus without cervix. There were 10 cases (58.8%) had normal endometrium, 3 cases (17.6%) had cystic space in adnexa area, 2 cases (11.8%) had malformation of renal duplication.

Conclusions

No vaginal gas line sonogram is the main ultrasound manifestation of patients with MRKH syndrome, and when having ultrasound examination, attention should be paid to whether they have other malformations or not. Abdominal ultrasonography could provide a imaging basis for accurate diagnosis evidences and classification of patients with MRKH syndrome.

Key words: Mullerian aplasia, Gynatresia, congenital, Female urogenital diseases, Ultrasonography, Menarche, Teen-age girls
图1 MRKH综合征超声检查的特征性表现(无阴道气体线回声)
表1 17例MRKH综合征患者腹部超声检查结果[例数(%)]
腹部超声检查结果 所占比例 腹部超声检查结果 所占比例
双侧始基子宫，无阴道 4(23.5) 双子宫，无宫颈，无阴道 1(5.9)
子宫发育正常，无阴道 6(35.3) 功能性子宫内膜 10(58.8)
正常宫体，无宫颈，无阴道 3(17.6) 子宫肌瘤 2(11.8)
单侧始基子宫，无宫颈，无阴道 2(11.8) 附件区囊性占位 3(17.6)
无子宫，无阴道 1(5.9) 左肾重复肾 2(11.8)
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